Diffuse histiocytic lymphoma complicating chronic lymphocytic leukemia

Armitage, Jamés O.; Dick, Fred R.; Corder, Michael P.

doi:10.1002/1097-0142(197802)41:2<422::aid-cncr2820410207>3.0.co;2-x

Cited by 102 publications

(35 citation statements)

References 12 publications

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“…Furthermore, CLL B cells are believed to be the transformed counterparts of normal B-1 cells. Transformation into high-grade large cell non-Hodgkin's lymphoma (NHL), designated Richter's syndrome, occurs in ϳ3-10% of CLL patients (49,50). In most cases, the high-grade component is considered to represent a blastic transformation of the CLL cells (51).…”

Section: Discussionmentioning

confidence: 99%

Latent Membrane Protein 2A, a Viral B Cell Receptor Homologue, Induces CD5+ B-1 Cell Development

Ikeda

Merchant

Lev

et al. 2004

The Journal of Immunology

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The latent membrane protein 2A (LMP2A) of EBV plays a key role in regulating viral latency and EBV pathogenesis by functionally mimicking a constitutively active B cell Ag receptor. When expressed as a B cell-specific transgene in mice, LMP2A drives B cell development, resulting in the bypass of normal developmental checkpoints. In this study, we have demonstrated that expression of LMP2A in transgenic mice results in B cell development that exclusively favors B-1 cells. This switch to B-1 cell development occurs at the pre-B-cell stage of normal B cell development in the bone marrow, a B cell stage much earlier than appreciated for B-1 commitment. This finding indicates that all pre-B cells have the capacity to assume a B-1 cell phenotype if they encounter the appropriate signal during normal development. Furthermore, these studies offer insight into EBV latency and pathogenesis in the human host.

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Section: Discussionmentioning

confidence: 99%

Latent Membrane Protein 2A, a Viral B Cell Receptor Homologue, Induces CD5+ B-1 Cell Development

Ikeda

Merchant

Lev

et al. 2004

The Journal of Immunology

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“…Classically, RS is pathologically represented by DLBCL [3,4,[9][10][11][34][35][36][37][38]. The disease involves most frequently the lymph nodes, but extra-nodal localizations are also not uncommon, and have been reported in the gastrointestinal tract, skin, liver, tonsil and bone marrow among other sites [3,4,[9][10][11][34][35][36][37][38].…”

Section: Histopathology Richter Syndromementioning

confidence: 99%

Richter syndrome: molecular insights and clinical perspectives

Rossi

Gaïdano

2009

Hematological Oncology

108

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Richter syndrome (RS) represents the clinico-pathologic transformation of chronic lymphocytic leukaemia (CLL) to an aggressive lymphoma, most commonly diffuse large B-cell lymphoma (DLBCL). The clinical definition of RS is heterogeneous, and encompasses at least two biologically different conditions: (i) CLL transformation to a clonally related DLBCL, that accounts for the majority of cases; (ii) development of a DLBCL unrelated to the CLL clone. In clonally related RS, the pathogenetic link between the CLL and the DLBCL phases is substantiated by the acquisition of novel molecular lesions at the time of clinico-pathologic transformation. RS is not a rare event in the natural history of CLL, since the cumulative incidence of RS at 10 years exceeds 10%. Prompt recognition of RS is known to be clinically useful, and may be favoured by close monitoring of CLL patients harbouring clinical and/or biological risk factors of RS development. Conventional risk factors that are independent predictors of RS development at the time of CLL diagnosis include: (i) expression of CD38; (ii) absence of del13q14 and (iii) lymph node size 3 cm. Other risk factors of RS development include CD38 genotype and usage of specific immunoglobulin variable genes. The molecular pathogenesis of RS has been elucidated to a certain extent. Acquisition of TP53 mutations and/or 17p13 deletion is a frequent molecular event in RS, as it is in other types of transformation from indolent to aggressive B-cell malignancies. Additional molecular alterations are being revealed by genome wide studies. Once that transformation has occurred, RS prognosis may be predicted by the RS score, based on performance status, LDH, platelet count, tumour size and number of prior therapies. Depending on patient's age and RS score, the therapeutic options for RS may range from conventional immunochemotherapy to allogeneic bone marrow transplantation.

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“…Occasionally, a subset of CLL cases acquires an increased number (10-55%) of PLs (CLL/PL) and eventually transforms to a neoplasm composed predominantly of PLs (5 (6). Transformation into a fast-growing diffuse large B-cell lymphoma is encountered in ~5% of CLL patients and it is referred to as Richter's syndrome (7,8). Under rare circumstances, plasmablastic lymphoma or plasmablastic transformation may be observed, representing an unusual example of Richter's syndrome.…”

Section: Introductionmentioning

confidence: 99%

Immunoglobulin gene translocations in chronic lymphocytic leukemia: A report of 35 patients and review of the literature

Braekeleer

Tous

Guéganic

et al. 2016

Molecular and Clinical Oncology

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Abstract. Chronic lymphocytic leukemia (CLL) represents the most common hematological malignancy in Western countries, with a highly heterogeneous clinical course and prognosis. Translocations involving the immunoglobulin (IG) genes are regularly identified. From 2000 to 2014, we identified an IG gene translocation in 18 of the 396 patients investigated at diagnosis (4.6%) and in 17 of the 275 analyzed during follow-up (6.2%). A total of 4 patients in whom the IG translocation was identified at follow-up did not carry the translocation at diagnosis. The IG heavy locus (IGH) was involved in 27 translocations (77.1%), the IG κ locus (IGK) in 1 (2.9%) and the IG λ locus (IGL) in 7 (20.0%). The chromosome band partners of the IG translocations were 18q21 in 16 cases (45.7%), 11q13 and 19q13 in 4 cases each (11.4% each), 8q24 in 3 cases (8.6%), 7q21 in 2 cases (5.7%), whereas 6 other bands were involved once (2.9% each). At present, 35 partner chromosomal bands have been described, but the partner gene has solely been identified in 10 translocations. CLL associated with IG gene translocations is characterized by atypical cell morphology, including plasmacytoid characteristics, and the propensity of being enriched in prolymphocytes. The IG heavy chain variable region (IGHV) mutational status varies between translocations, those with unmutated IGHV presumably involving cells at an earlier stage of B-cell lineage. All the partner genes thus far identified are involved in the control of cell proliferation and/or apoptosis. The translocated partner gene becomes transcriptionally deregulated as a consequence of its transposition into the IG locus. With the exception of t(14;18)(q32;q21) and its variants, prognosis appears to be poor for the other translocations.Therefore, searching for translocations involving not only IGH, but also IGL and IGK, by banding and molecular cytogenetics is required. Furthermore, it is important to identify the partner gene to ensure the patients receive the optimal treatment.

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Diffuse histiocytic lymphoma complicating chronic lymphocytic leukemia

Cited by 102 publications

References 12 publications

Latent Membrane Protein 2A, a Viral B Cell Receptor Homologue, Induces CD5+ B-1 Cell Development

Latent Membrane Protein 2A, a Viral B Cell Receptor Homologue, Induces CD5+ B-1 Cell Development

Richter syndrome: molecular insights and clinical perspectives

Immunoglobulin gene translocations in chronic lymphocytic leukemia: A report of 35 patients and review of the literature

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